TY - JOUR
T1 - Myasthenia gravis
AU - Gilhus, Nils Erik
AU - Tzartos, Socrates
AU - Evoli Stampanoni-B, Amelia
AU - Palace, Jacqueline
AU - Burns, Ted M.
AU - Verschuuren, Jan J. G. M.
PY - 2019
Y1 - 2019
N2 - Abstract
Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thymectomy and immunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.
AB - Abstract
Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thymectomy and immunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.
KW - Acetylcholinesterase
KW - Adrenal Cortex Hormones
KW - Agrin
KW - Anti-Inflammatory Agents, Non-Steroidal
KW - Autoantibodies
KW - Biomarkers
KW - Blepharoptosis
KW - Collagen
KW - Cortactin
KW - Electromyography
KW - Humans
KW - Kv1.4 Potassium Channel
KW - LDL-Receptor Related Proteins
KW - Muscle Proteins
KW - Myasthenia Gravis
KW - Receptor Protein-Tyrosine Kinases
KW - Receptors, Cholinergic
KW - Receptors, Nicotinic
KW - Risk Factors
KW - Ryanodine Receptor Calcium Release Channel
KW - Acetylcholinesterase
KW - Adrenal Cortex Hormones
KW - Agrin
KW - Anti-Inflammatory Agents, Non-Steroidal
KW - Autoantibodies
KW - Biomarkers
KW - Blepharoptosis
KW - Collagen
KW - Cortactin
KW - Electromyography
KW - Humans
KW - Kv1.4 Potassium Channel
KW - LDL-Receptor Related Proteins
KW - Muscle Proteins
KW - Myasthenia Gravis
KW - Receptor Protein-Tyrosine Kinases
KW - Receptors, Cholinergic
KW - Receptors, Nicotinic
KW - Risk Factors
KW - Ryanodine Receptor Calcium Release Channel
UR - http://hdl.handle.net/10807/148578
U2 - 10.1038/s41572-019-0079-y
DO - 10.1038/s41572-019-0079-y
M3 - Article
SN - 2056-676X
VL - 5
SP - 30-N/A
JO - NATURE REVIEWS. DISEASE PRIMERS
JF - NATURE REVIEWS. DISEASE PRIMERS
ER -